Some people do not need any treatment when they are first diagnosed. WM usually develops slowly, the term to describe this gradual behaviour is 'indolent'. This means some people may not need treatment for many months, often years. During this time you will have regular check-ups in the outpatients clinic. This active monitoring is known as being on 'watch and wait' or 'watchful waiting'.
If your doctor decides no treatment is needed you will have regular check-ups, to assess how you are feeling and to take blood tests to measure your blood cell counts and IgM levels. This kind of follow-up, with check-ups but without treatment, is quite common in people with WM. If you have no symptoms you will typically be seen in the clinic every 3-6 months for review.
Watch and wait may feel difficult but can often be the best treatment for WM initially. There is no evidence that starting treatment early is advantageous. It can be hard to wait for symptoms to develop or for things to get worse before anything is done. It can make you feel anxious and unable to enjoy the relative good health you have. If this happens, it is important to talk about this with your doctor, specialist nurse and family and friends as there is support available, including psychological support.
Treatment is aimed at improving your quality of life and keeping you well for as long as possible, with the least possible side effects.
Your treatment may start if:
- You begin to get symptoms that are due to your WM
- The level of IgM protein in your blood is increasing at a rapid rate or you experience hyperviscosity (thickening of the blood)
- Your blood count changes - for example, you develop anaemia (low levels of red blood cells)
- You develop other complications, such as a progressive neuropathy, cryoglobulinaemia or Cold Agglutinin Disease, that is felt to be due to the effects of the abnormal IgM levels.
The treatment you have will depend on your personal situation. Your medical team will assess you based on:
- The results of all your tests
- Your symptoms - for example, how severe they are and whether you have neuropathy
- Your age and general health
There are a range of treatments available for WM. The main treatment has for many years been chemotherapy. However, as a result of research and clinical trials, many new therapies are becoming available to treat WM patients, often known as 'biological treatments'.
Chemotherapy (chemo) is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It can be given as tablets, into a vein (intravenously), or as an injection under the skin (subcutaneously).
Steroids are often used as part of the treatment as they can help the other drugs to destroy the abnormal B-cells and make chemotherapy more effective.
Monoclonal antibodies are drugs that recognise, target and stick to particular proteins on the surface of cancer cells. They can stimulate the body’s own immune system to destroy these cells.
Most patients with symptomatic WM will received a combination of rituximab (a biological therapy), plus chemotherapy. The exact choice of regimen depends on multiple factors, including the level of bone marrow involvement, whether there is hyperviscosity or neuropathy, the age and fitness of the patient, and if autologous stem cell transplantation is planned.
A great deal of international collaboration and effort in recent years means that there are new therapies becoming available for patients with WM. This includes agents which target chemical pathways within the lymphoplasmacytic (LPL) cells and also affect the way LPL cells collaborate with the environment in which they live (the bone marrow microenvironment). Others include ‘immunomodulatory’ drugs such as thalidomide.
Some people with WM may have treatment involving the use of their own stem cells (autologous stem cell transplant or ASCT), or stem cells from a donor (allogeneic stem cell transplant or allo-SCT).
This is a treatment in which a patient's T-cells (a type of immune system cell) are changed in the laboratory so they will attack cancer cells.
Supportive treatments are designed to counteract some of the symptoms of the lymphoma and the side effects of the treatments. In WM, these supportive treatments include antibiotics that are given to prevent infections (which may occur during chemotherapy cycles), blood transfusions and plasmapheresis.
Clinical trials are research studies that test new medical treatments and you may be asked if you would like to take part in one. Clinical trials are very important in improving future treatments for people with WM and also provide you with a chance to access new treatments that aren’t available elsewhere.